摘要目的 探讨血管内大B细胞淋巴瘤(IVLBCL)的临床病理特征及病理诊断.方法 对2例IVLBCL的尸体解剖检查(简称尸检)病例进行了临床病理、免疫表型(SP法染色)及抗原受体基因(TCRγ、IgH)重排检测.结果 (1)一般情况:2例尸检分别于2005年和1979年进行,均为男性患者,年龄为70岁和50岁.2人均以神经系统功能障碍(双下肢麻木或无力)为首发表现,起病急,病程短(分别为30 d和20 d),病情进展迅速,并在短期内出现多器官功能衰竭而死亡,生存期分别为85 d和44 d.(2)尸检主要发现:除例1患者有多发性皮肤和左心外膜下结节(直径0.5～2.5 cm)外,全身均未见明显占位性病变,但双肺、肾脏、脑、脾脏等脏器有不同程度地均匀肿大和重量增加.例1患者还有多浆膜腔积液.组织学观察见多脏器、组织(脑、垂体、脊髓和脊神经根、心、肺、肾、肝、脾、消化管、淋巴结、胰、肾上腺、甲状腺、睾丸等)的小血管内见数量不等的异形淋巴样细胞存在,且各处之肿瘤细胞形态相似.瘤细胞中等偏大,呈圆形或卵圆形,细胞质丰富,核质比高.细胞核为圆形或卵圆形,核膜较厚,核染色质呈斑块状散在分布,可见1～3个嗜碱性的小核仁.(3)免疫表型及抗原受体基因重排检测:肿瘤细胞均表达B淋巴细胞分化抗原CD20和CD79a;部分瘤细胞表达bcl-2蛋白和CD5抗原;2例之肿瘤细胞均不表达T细胞及髓系分化抗原等[CD3、髓过氧化物酶(MPO)、bcl-6、CD30、CD10和PCK等];Ki-67阳性率均为80%.PCR检测均检出免疫球蛋白重链基因(IgH)克隆性重排,而TCRγ基因呈胚系构型.结论 2例IVLBCL均以神经系统功能障碍为首发表现,有相似的病理形态学特征,免疫表型及基因型,预后差.

abstractsObjective To study the clinicopathologic features of intravascular large B-cell lymphoma (IVLBCL).Methods Two autopsy cases of IVLBCL were retrieved from the archival file.The clinicopathologic features,immunohistochemistry and molecular findings were studied. Results The deceased were 70-year-old and 50-year-old males.Both of them had complained of a sudden onset of weakness and numnbness of lower extremities.The clinical course deteriorated rapidly,with multi-organ failure.They died 85 days and 44 days after the presentation,respectively.Post-modem examination did not reveal any mass lesion,except the presence of multiple skin and epicardium nodules,ranging from 0.5 cm to 2.5 cm in diameter,in the first patient.Pericardial effusion.ascites and pleural effusion were also observed.Histologically,neoplastic lymphoid cells filled up the small vessel lumina in many organs,including brain,hypophysis,spinal cord,spinal nerve roots,heart,lungs,kidneys,liver,spleen,digestive tract,pancreas,adrenal,thyroid,testes and lymph nodes.The tumor cells were relatively monotonous and of medium to large in size with round vesicular nuclei and 1 to 3 small basophilic nucleoli.Immunohistochemical study showed that the lymphoma cells expressed B-cell markers CD20 and CD79a,occasionally positive for CD5 and bcl-2 but negative for CD3,bcl-6,CDI0,CD30,myeloperoxidase and cytokeratin.In-situ hybridization for Epstein-Barr virus-encoded RNA wag negative.The proliferative index,as demonstrated by Ki-67 staining,was about 80%.Molecular study showed the presence of immunoglobulin heavy chain gene rearrangement in both cases,T-cell receptor-γ gene rearrangement was not found.Conclusions IVLBCL may present as neurological disturbance and carries distinctive morphologiccharacteristics,immunophenotype and molecular findings.The prognosis of this disease is often dismal.